Related Experiment Videos
OMENS-Plus: analysis of craniofacial and extracraniofacial anomalies in hemifacial microsomia
J E Horgan1, B L Padwa, R A LaBrie
1Harvard School of Dental Medicine, Boston, Massachusetts, USA.
Insights
Hemifacial microsomia (HFM) frequently occurs with extracraniofacial anomalies, particularly CNS, cardiac, and skeletal issues. The OMENS classification may be expanded to OMENS-Plus to include these associated conditions.
Area of Science:
- Craniofacial Surgery
- Genetics
- Pediatric Medicine
Background:
- Hemifacial microsomia (HFM) is a congenital condition affecting facial development.
- The spectrum of HFM can include extracraniofacial anomalies, impacting multiple organ systems.
- Understanding these associations is crucial for comprehensive patient management.
Purpose of the Study:
- To investigate the prevalence and types of extracraniofacial anomalies in a cohort of hemifacial microsomia patients.
- To identify associations between craniofacial features and extracraniofacial anomalies using the OMENS classification.
- To propose an updated classification system for HFM that incorporates extracraniofacial involvement.
Main Methods:
- Retrospective review of 121 patients diagnosed with hemifacial microsomia.
- Detailed analysis of craniofacial features using the Orbital, Mandibular, Ear, Neural, and Soft tissue (OMENS) grading system.
- Statistical analysis to determine associations between OMENS variables and extracraniofacial anomalies.
Main Results:
- Over half (55.4%) of HFM patients exhibited extracraniofacial anomalies, with 42.4% having multiple system involvement.
- Central nervous system, cardiac, and skeletal anomalies were significantly associated with HFM.
- Higher OMENS grades correlated with the presence and severity of extracraniofacial structural defects.
Conclusions:
- HFM is frequently associated with extracraniofacial anomalies, supporting a potential neural crest developmental pathway.
- The OMENS classification system is valuable for predicting extracraniofacial involvement in HFM.
- An expanded OMENS-Plus classification is proposed to better represent the full spectrum of HFM, including extracraniofacial anomalies.
Abstract:
This review of 121 patients with hemifacial microsomia (HFM) revealed that 67 (55.4%) had extracraniofacial anomalies. Sixteen patients (13%) had one extracraniofacial anomaly and 51 patients (42.4%) had anomalies of multiple organ systems. There was no gender or side predominance in the cohort with the HFM "expanded spectrum." Central nervous system (CNS), cardiac, and skeletal anomalies were "associated" (i.e., had frequencies of 10% or more). Pulmonary, gastrointestinal, and renal deformities were equivocally associated. Statistical analysis indicated significant associations between several orbital, mandibular, ear, neural, and soft tissue (OMENS) variables and extracraniofacial anomalies. Patients with extracraniofacial structural defects had higher OMENS grades for individual craniofacial anatomic categories. Furthermore, patients with expanded spectrum had higher total OMENS scores. The frequency of cardiac anomalies (26%) supports the model of neural crest involvement in the pathogenesis of both hemifacial microsomia and conotruncal defects. The majority of the heart defects in this study were of either the outflow or septal type. We propose that the OMENS classification system for craniofacial anomalies of HFM be expanded to OMENS-Plus (+) to designate the presence of associated extracraniofacial anomalies.